PABPN1 blocking peptide

PABPN1 Antibody (C-term) Blocking Peptide

• Gene Names: PABPN1; OPMD; PAB2; PABII; PABP2; PABP-2
• Synonyms: PABPN1; PABPN1 Antibody (C-term) Blocking Peptide; Polyadenylate-binding protein 2; PABP-2; Poly(A)-binding protein 2; Nuclear poly(A)-binding protein 1; Poly(A)-binding protein II; PABII; Polyadenylate-binding nuclear protein 1; PAB2; PABP2; PABPN1 blocking peptide

• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 306

• Cellular Location: Nucleus. Cytoplasm Note: Localized in cytoplasmic mRNP granules containing untranslated mRNAs. Shuttles between the nucleus and the cytoplasm but predominantly found in the nucleus. Its nuclear import may involve the nucleocytoplasmic transport receptor transportin and a RAN-GTP-sensitive import mechanism. Is exported to the cytoplasm by a carrier-mediated pathway that is independent of mRNA traffic Nucleus; nuclear speckle. Colocalizes with SKIP and poly(A) RNA in nuclear speckles (By similarity).
• Tissue Location: Ubiquitous.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for PABPN1 blocking peptide

Involved in the 3'-end formation of mRNA precursors (pre-mRNA) by the addition of a poly(A) tail of 200-250 nt to the upstream cleavage product. Stimulates poly(A) polymerase (PAPOLA) conferring processivity on the poly(A) tail elongation reaction and controls also the poly(A) tail length. Increases the affinity of poly(A) polymerase for RNA. Is also present at various stages of mRNA metabolism including nucleocytoplasmic trafficking and nonsense-mediated decay (NMD) of mRNA. Cooperates with SKIP to synergistically activate E-box-mediated transcription through MYOD1 and may regulate the expression of muscle-specific genes. Binds to poly(A) and to poly(G) with high affinity. May protect the poly(A) tail from degradation (By similarity).

NCBI and Uniprot Product Information

• NCBI GI #: 46403176
• NCBI GeneID: 8106
• UniProt Accession #: Q86U42; O43484; D3DS49
• Molecular Weight: 37,171 Da
• NCBI Official Full Name: Polyadenylate-binding protein 2
• NCBI Official Synonym Full Names: poly(A) binding protein nuclear 1
• NCBI Official Symbol: PABPN1
• NCBI Official Synonym Symbols: OPMD; PAB2; PABII; PABP2; PABP-2
• NCBI Protein Information: polyadenylate-binding protein 2
• UniProt Protein Name: Polyadenylate-binding protein 2
• UniProt Gene Name: PABPN1
• UniProt Synonym Gene Names: PAB2; PABP2; PABP-2; Poly(A)-binding protein 2; PABII
• UniProt Entry Name: PABP2_HUMAN

NCBI Description

This gene encodes an abundant nuclear protein that binds with high affinity to nascent poly(A) tails. The protein is required for progressive and efficient polymerization of poly(A) tails at the 3' ends of eukaryotic transcripts and controls the size of the poly(A) tail to about 250 nt. At steady-state, this protein is localized in the nucleus whereas a different poly(A) binding protein is localized in the cytoplasm. This gene contains a GCG trinucleotide repeat at the 5' end of the coding region, and expansion of this repeat from the normal 6 copies to 8-13 copies leads to autosomal dominant oculopharyngeal muscular dystrophy (OPMD) disease. Related pseudogenes have been identified on chromosomes 19 and X. Read-through transcription also exists between this gene and the neighboring upstream BCL2-like 2 (BCL2L2) gene. [provided by RefSeq, Dec 2010]

Uniprot Description

PABPN1: Involved in the 3'-end formation of mRNA precursors (pre-mRNA) by the addition of a poly(A) tail of 200-250 nt to the upstream cleavage product. Stimulates poly(A) polymerase (PAPOLA) conferring processivity on the poly(A) tail elongation reaction and controls also the poly(A) tail length. Increases the affinity of poly(A) polymerase for RNA. Is also present at various stages of mRNA metabolism including nucleocytoplasmic trafficking and nonsense-mediated decay (NMD) of mRNA. Cooperates with SKIP to synergistically activate E-box-mediated transcription through MYOD1 and may regulate the expression of muscle-specific genes. Binds to poly(A) and to poly(G) with high affinity. May protect the poly(A) tail from degradation. Defects in PABPN1 are the cause of oculopharyngeal muscular dystrophy (OPMD). OPMD is a form of late- onset slowly progressive myopathy characterized by eyelid ptosis, dysphagia and, sometimes by other cranial and limb-muscle involvement. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA splicing; RNA-binding

Chromosomal Location of Human Ortholog: 14q11.2

Cellular Component: nucleoplasm; nucleus; ribonucleoprotein complex

Molecular Function: protein binding; RNA binding

Biological Process: modification by virus of host mRNA processing; mRNA 3'-end processing; muscle contraction; nuclear mRNA splicing, via spliceosome; poly(A)+ mRNA export from nucleus; RNA processing; termination of RNA polymerase II transcription

Disease: Oculopharyngeal Muscular Dystrophy